Beatrice Latal

Psychological adjustment and quality of life in children and adolescents following open-heart surgery for congenital heart disease: a systematic review

BackgroundChildren with congenital heart defects (CHD) requiring open-heart surgery are a group at high risk for health-related sequelae. Little consensus exists regarding their long-term psychological adjustment (PA) and health-related quality of life (QoL). Thus, we conducted a systematic review to determine the current knowledge on long-term outcome in this population.MethodsWe included randomized controlled trials, case control, or cohort studies published between 1990–2008 evaluating self- and proxy-reported PA and QoL in patients aged between two and 17 years with a follow-up of at least two years after open heart surgery for CHD.ResultsTwenty-three studies assessing psychological parameters and 12 studies assessing QoL were included. Methodological quality of the studies varied greatly with most studies showing a moderate quality. Results were as follows: (a) A considerable proportion of children experienced psychological maladjustment according to their parents; (b) studies on self-reported PA indicate a good outcome; (c) the studies on QoL suggest an impaired QoL for some children in particular for those with more severe cardiac disease; (d) parental reports of psychological maladjustment were related to severity of CHD and developmental delay.ConclusionA significant proportion of survivors of open-heart surgery for CHD are at risk for psychological maladjustment and impaired QoL. Future research needs to focus on self-reports, QoL data and adolescents.

Neurodevelopmental Outcomes After Cardiac Surgery in Infancy

BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI). RESULTS: Among 1770 subjects from 22 institutions, assessed at age 14.5 ± 3.7 months, PDIs and MDIs (77.6 ± 18.8 and 88.2 ± 16.7, respectively) were lower than normative means (each P < .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P ≤ .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P < .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02). CONCLUSIONS: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources.

Clinical signs predict 30-month neurodevelopmental outcome after neonatal encephalopathy

OBJECTIVE This study was undertaken to determine the value of a neonatal encephalopathy score (ES) and the presence of seizures for predicting 30-month neurodevelopmental outcome. STUDY DESIGN In a cohort study, 68 term newborn infants with encephalopathy were evaluated with an ES based on alertness, feeding, tone, respiratory status, reflexes, and seizure activity (range: 0-6). Seizures were noted as present or absent clinically. Significant cognitive deficits (Mental Development Index <70), motor disability (spastic triplegia/quadriplegia), or death were abnormal outcomes. RESULTS Twenty-two newborn infants (32%) had abnormal outcomes. With the use of maximum ES and presence of seizures from days 1 to 3 of life, 87% of newborn infants were correctly classified (area under receiver operating curve 0.93). By using ES and presence of seizures on day 1 only, 87% of newborn infants were correctly classified (area under receiver operating curve 0.89). CONCLUSION The severity of neonatal encephalopathy and the presence of seizures are valuable predictors of 30-month neurodevelopmental outcome, as early as the first day of life.

Children With Congenital Hypothyroidism: Long-Term Intellectual Outcome After Early High-Dose Treatment

We aim to determine long-term intellectual outcome of adolescents with early high-dose treated congenital hypothyroidism (CH). Sixty-three prospectively followed children with CH were assessed at age of 14 y with the Wechsler Intelligence Scale for Children-Revised and compared with 175 healthy controls. Median age at onset of treatment was 9 d (range 5–18 d) and median starting dose of levothyroxine (L-T4) was 14.7 μg/kg/d (range 9.9–23.6 μg/kg/d). Full-scale intelligence quotient (IQ) was significantly lower than in controls after adjustment for socioeconomic status (SES) and gender (101.7 versus 111.4; p < 0.0001). Children with athyreosis had a lower performance IQ than those with dysgenesis (adjusted difference 7.6 IQ scores, p < 0.05). Lower initial thyroxine (T4) levels correlated with poorer IQ (r = 0.27, p = 0.04). Lower SES was associated with poorer IQ, in particular in children with CH (interaction, p = 0.03). Treatment during childhood was not related to IQ at age 14 y. Adolescents with CH manifest IQ deficits when compared with their peers despite early high-dose treatment and optimal substitution therapy throughout childhood. Those adolescents with athyreosis and lower SES are at particular risk for adverse outcome. Therefore, early detection of intellectual deficits is mandatory in children with CH.

Brain volumes predict neurodevelopment in adolescents after surgery for congenital heart disease

Patients with complex congenital heart disease are at risk for neurodevelopmental impairments. Evidence suggests that brain maturation can be delayed and pre- and postoperative brain injury may occur, and there is limited information on the long-term effect of congenital heart disease on brain development and function in adolescent patients. At a mean age of 13.8 years, 39 adolescent survivors of childhood cardiopulmonary bypass surgery with no structural brain lesions evident through conventional cerebral magnetic resonance imaging and 32 healthy control subjects underwent extensive neurodevelopmental assessment and cerebral magnetic resonance imaging. Cerebral scans were analysed quantitatively using surface-based and voxel-based morphometry. Compared with control subjects, patients had lower total brain (P = 0.003), white matter (P = 0.004) and cortical grey matter (P = 0.005) volumes, whereas cerebrospinal fluid volumes were not different. Regional brain volume reduction ranged from 5.3% (cortical grey matter) to 11% (corpus callosum). Adolescents with cyanotic heart disease showed more brain volume loss than those with acyanotic heart disease, particularly in the white matter, thalami, hippocampi and corpus callosum (all P-values < 0.05). Brain volume reduction correlated significantly with cognitive, motor and executive functions (grey matter: P < 0.05, white matter: P < 0.01). Our findings suggest that there are long-lasting cerebral changes in adolescent survivors of cardiopulmonary bypass surgery for congenital heart disease and that these changes are associated with functional outcome.

Outcome at two years of age in a Swiss national cohort of extremely preterm infants born between 2000 and 2008

BackgroundWhile survival rates of extremely preterm infants have improved over the last decades, the incidence of neurodevelopmental disability (ND) in survivors remains high. Representative current data on the severity of disability and of risk factors associated with poor outcome in this growing population are necessary for clinical guidance and parent counselling.MethodsProspective longitudinal multicentre cohort study of preterm infants born in Switzerland between 240/7 and 276/7 weeks gestational age during 2000–2008. Mortality, adverse outcome (death or severe ND) at two years, and predictors for poor outcome were analysed using multilevel multivariate logistic regression. Neurodevelopment was assessed using Bayley Scales of Infant Development II. Cerebral palsy was graded after the Gross Motor Function Classification System.ResultsOf 1266 live born infants, 422 (33%) died. Follow-up information was available for 684 (81%) survivors: 440 (64%) showed favourable outcome, 166 (24%) moderate ND, and 78 (11%) severe ND. At birth, lower gestational age, intrauterine growth restriction and absence of antenatal corticosteroids were associated with mortality and adverse outcome (p < 0.001). At 360/7 weeks postmenstrual age, bronchopulmonary dysplasia, major brain injury and retinopathy of prematurity were the main predictors for adverse outcome (p < 0.05). Survival without moderate or severe ND increased from 27% to 39% during the observation period (p = 0.02).ConclusionsIn this recent Swiss national cohort study of extremely preterm infants, neonatal mortality was determined by gestational age, birth weight, and antenatal corticosteroids while neurodevelopmental outcome was determined by the major neonatal morbidities. We observed an increase of survival without moderate or severe disability.

Impaired motor performance and movement quality in very-low-birthweight children at 6 years of age

Motor performance and movement quality were quantitatively examined (Zurich Neuromotor Assessment: timed motor performances and associated movements) in 87 prospectively enrolled very-low-birthweight (VLBW; <1250g) children (38 males, 49 females; mean birthweight 1016.2g [SD 141.5]:, range 720-1240g; mean gestational age 28.7wks [SD 2], range 25.7-33.4wks) at 6 years of age. All motor tasks were below the reference population: pure motor (median z-score) -0.46; adaptive fine motor (pegboard) -0.99; adaptive gross motor -0.88; static balance -0.48; and associated movements -1.90. All tasks correlated with the degree of neurological abnormalities (p<or=0.004). VLBW children with no neurological abnormality also performed below the 10th centile and associated movements occurred more frequently than in the reference population (odds ratio 18, 95% confidence interval 6.7-47.9). Severity of periventricular leukomalacia and intraventricular haemorrhage assessed by ultrasound was associated with adaptive fine and gross motor tasks. We conclude that speed of motor performance and movement quality in particular were substantially impaired in VLBW children and are related to the degree of neurological abnormalities and neonatal cerebral injury.

Prediction of neurodevelopmental outcome after preterm birth.

Prediction of outcome after preterm birth is critical, but remains difficult, particularly in the early postnatal period. The ability to predict outcome improves parental counseling and selection of infants for early therapeutic strategies aiming at preventing or ameliorating cerebral injury. This review gives an overview of the spectrum and severity of neurodevelopmental, behavioral, and psychosocial outcomes, with discussion of predictors of outcome and, in particular, the clinical, electrophysiological, and imaging predictors. A detailed neurologic examination of infants is a valuable predictive tool in terms of later moderate to severe neurodevelopmental impairments; however, it may be limited in the immediate newborn period. Electrophysiological, neuroimaging, and clinical risk factors for adverse neurodevelopmental outcome have been identified. Good prediction is usually achieved for major functional disabilities in early childhood, but is poorer for moderate or mild long-term outcome. Future research should focus on the long-term quality of life, academic achievement, and the influence of the sociocultural environment. More emphasis should be placed on genetic diversity as a modifying factor for the large variability in outcome.

Surgery-related posttraumatic stress disorder in parents of children undergoing cardiopulmonary bypass surgery: a prospective cohort study.

Objective: We aimed at evaluating surgery-related posttraumatic stress disorder (PTSD) in parents of children undergoing cardiopulmonary bypass surgery. Risk factors for parental PTSD symptoms were explored. Design: A prospective cohort study was performed assessing PTSD symptoms immediately after discharge and 6 months after cardiopulmonary bypass surgery. Setting: Recruitment took place at a tertiary pediatric medical center in Switzerland. Subjects: German-speaking parents of children with congenital heart defects aged between 0 and 16 yrs undergoing cardiopulmonary bypass surgery were eligible (n = 228). After child discharge, 135 mothers and 98 fathers of 139 children (response rate 61.0%) participated. Six months after surgery, 121 mothers and 92 fathers of 128 children (response rate, 56.1%) took part in the study. Interventions: Assessment via a screening instrument and self-rating scale, and extraction of data from charts. Measurements and Main Results: The Posttraumatic Diagnostic Scale was applied to estimate self-reported symptoms of PTSD. Following discharge, 16.4% of mothers and 13.3% of fathers met diagnostic criteria for acute PTSD. Another 15.7% of mothers and 13.3% of fathers experienced significant symptoms of posttraumatic stress. Six months after surgery, PTSD rates were 14.9% and 9.5%, respectively. Mothers experienced more severe symptoms of PTSD, but gender differences were not detected with regard to the frequency of PTSD at either time. After controlling for socioeconomic status and child preoperative morbidity, PTSD symptom severity after discharge remained the only significant predictor of PTSD severity at 6 months. Pre-, peri-, and postoperative factors did not predict parental PTSD. Conclusions: Parents of children undergoing cardiopulmonary bypass surgery are at increased risk for intermediate and long-term psychological malfunctioning. Acute symptoms of PTSD in parents shortly after discharge of their child are a major risk factor for the development of chronic PTSD. Clinicians need to identify parents at risk at an early stage to provide them with systematic support.

Early, Accurate Diagnosis and Early Intervention in Cerebral Palsy: Advances in Diagnosis and Treatment

Importance Cerebral palsy describes the most common physical disability in childhood and occurs in 1 in 500 live births. Historically, the diagnosis has been made between age 12 and 24 months but now can be made before 6 months’ corrected age. Objectives To systematically review best available evidence for early, accurate diagnosis of cerebral palsy and to summarize best available evidence about cerebral palsy–specific early intervention that should follow early diagnosis to optimize neuroplasticity and function. Evidence Review This study systematically searched the literature about early diagnosis of cerebral palsy in MEDLINE (1956-2016), EMBASE (1980-2016), CINAHL (1983-2016), and the Cochrane Library (1988-2016) and by hand searching. Search terms included cerebral palsy, diagnosis, detection, prediction, identification, predictive validity, accuracy, sensitivity, and specificity. The study included systematic reviews with or without meta-analyses, criteria of diagnostic accuracy, and evidence-based clinical guidelines. Findings are reported according to the PRISMA statement, and recommendations are reported according to the Appraisal of Guidelines, Research and Evaluation (AGREE) II instrument. Findings Six systematic reviews and 2 evidence-based clinical guidelines met inclusion criteria. All included articles had high methodological Quality Assessment of Diagnostic Accuracy Studies (QUADAS) ratings. In infants, clinical signs and symptoms of cerebral palsy emerge and evolve before age 2 years; therefore, a combination of standardized tools should be used to predict risk in conjunction with clinical history. Before 5 months’ corrected age, the most predictive tools for detecting risk are term-age magnetic resonance imaging (86%-89% sensitivity), the Prechtl Qualitative Assessment of General Movements (98% sensitivity), and the Hammersmith Infant Neurological Examination (90% sensitivity). After 5 months’ corrected age, the most predictive tools for detecting risk are magnetic resonance imaging (86%-89% sensitivity) (where safe and feasible), the Hammersmith Infant Neurological Examination (90% sensitivity), and the Developmental Assessment of Young Children (83% C index). Topography and severity of cerebral palsy are more difficult to ascertain in infancy, and magnetic resonance imaging and the Hammersmith Infant Neurological Examination may be helpful in assisting clinical decisions. In high-income countries, 2 in 3 individuals with cerebral palsy will walk, 3 in 4 will talk, and 1 in 2 will have normal intelligence. Conclusions and Relevance Early diagnosis begins with a medical history and involves using neuroimaging, standardized neurological, and standardized motor assessments that indicate congruent abnormal findings indicative of cerebral palsy. Clinicians should understand the importance of prompt referral to diagnostic-specific early intervention to optimize infant motor and cognitive plasticity, prevent secondary complications, and enhance caregiver well-being.